イデュルスルファーゼ
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/01/28 22:55:19」(JST)
[Wiki en表示]
Idursulfase
| Clinical data |
| AHFS/Drugs.com |
monograph |
| Identifiers |
| CAS Number |
50936-59-9 Y |
| ATC code |
A16AB09 |
| DrugBank |
DB01271 Y |
| ChemSpider |
none |
| UNII |
5W8JGG2651 Y |
| KEGG |
D04499 Y |
| ChEMBL |
CHEMBL1201826 N |
|
| NY (what is this?) (verify) |
Idursulfase (brand name Elaprase), manufactured by Shire, is a drug used to treat Hunter syndrome (also called MPS-II).[1] It is a purified form of the lysosomal enzyme iduronate-2-sulfatase and is produced by recombinant DNA technology in a human cell line.
It is one of the most expensive drugs ever produced, costing US$375,000 per patient per year.[2][3]
References
- ^ Garcia AR, DaCosta JM, Pan J, Muenzer J, Lamsa JC (2007). "Preclinical dose ranging studies for enzyme replacement therapy with idursulfase in a knock-out mouse model of MPS II". Mol. Genet. Metab. 91 (2): 183–90. doi:10.1016/j.ymgme.2007.03.003. PMID 17459751.
- ^ "Drug approved to treat rare but potentially deadly disease". Retrieved 2011-04-29.
- ^ Health Care: The World's Most Expensive Drugs, Matthew Herper, Forbes, Feb. 22, 2010
External links
- idursulfase at the US National Library of Medicine Medical Subject Headings (MeSH)
|
Other alimentary tract and metabolism products (A16)
|
|
| Amino acids and derivatives |
- Levocarnitine
- Ademetionine
- Levoglutamide
- Cysteamine
- Carglumic acid
- Betaine
|
|
| Enzymes |
- Carbohydrate metabolism: sucrase (Sacrosidase)
- alpha-glucosidase (Alglucosidase alfa)
- Glycolipid/sphingolipid: glucocerebrosidase (Alglucerase
- Imiglucerase
- Taliglucerase alfa
- Velaglucerase alfa)
- alpha-galactosidase (Agalsidase alfa
- Agalsidase beta)
- Glycosaminoglycan: iduronidase (Laronidase)
- arylsulfatase B (Galsulfase)
- iduronate-2-sulfatase (Idursulfase)
- Lipid: lysosomal lipase (Sebelipase alfa)
|
|
| Other |
- Anethole trithione
- Eliglustat
- Glycerol phenylbutyrate
- Miglustat
- Nitisinone
- Sapropterin
- Sodium benzoate
- Sodium phenylbutyrate
- Teduglutide
- Tioctic acid
- Zinc acetate
|
|
|
Index of digestion
|
|
| Description |
- Anatomy
- Physiology
- Development
|
|
| Disease |
- Congenital
- Neoplasms and cancer
- Inflammatory bowel disease
- Gluten sensitivity
- Other
- Symptoms and signs
- Blood tests
|
|
| Treatment |
- Procedures
- Drugs
- anabolic steroids
- antacids
- diarrhoea and infection
- bile and liver
- functional gastrointestinal disorders
- laxatives
- peptic ulcer and reflux
- nausea and vomiting
- other
- Surgery
|
|
|
UpToDate Contents
全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.
English Journal
- Intrafamilial variability in the clinical manifestations of mucopolysaccharidosis type II: Data from the Hunter Outcome Survey (HOS).
- Ficicioglu C1, Giugliani R2, Harmatz P3, Mendelsohn NJ4,5, Jego V6, Parini R7.
- American journal of medical genetics. Part A.Am J Med Genet A.2018 Feb;176(2):301-310. doi: 10.1002/ajmg.a.38551. Epub 2017 Dec 6.
- PMID 29210515
- Survival in idursulfase-treated and untreated patients with mucopolysaccharidosis type II: data from the Hunter Outcome Survey (HOS).
- Burton BK1, Jego V2, Mikl J3, Jones SA4.
- Journal of inherited metabolic disease.J Inherit Metab Dis.2017 Nov;40(6):867-874. doi: 10.1007/s10545-017-0075-x. Epub 2017 Sep 8.
- PMID 28887757
- Clinical outcomes in idursulfase-treated patients with mucopolysaccharidosis type II: 3-year data from the hunter outcome survey (HOS).
- Muenzer J1, Giugliani R2, Scarpa M3,4, Tylki-Szymańska A5, Jego V6, Beck M7.
- Orphanet journal of rare diseases.Orphanet J Rare Dis.2017 Oct 3;12(1):161. doi: 10.1186/s13023-017-0712-3.
- PMID 28974237
Japanese Journal
- Comparative study of idursulfase beta and idursulfase in vitro and in vivo
- An overview of Korean patients with mucopolysaccharidosis and collaboration through the Asia Pacific MPS Network
Related Links
- You should not receive this medication if you are allergic to idursulfase. Before receiving idursulfase, tell your doctor if you are allergic to any drugs, or if you have asthma or other breathing disorder. You may be more likely to have a ...
- Idursulfase - Idursulfase is used to treat some of the symptoms of a genetic condition called Hunter's syndrome. Hunter syndrome is also called mucopolysaccharidosis (MYOO-koe-pol-ee-SAK-a-rye-DOE-sis). - Idursulfase may ...
★リンクテーブル★
[★]
- 英
- idursulfase
- 商
- エラプレース
- 関
- 酵素製剤